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Global Study of Del-desiran for the Treatment of DM1

Phase 3
Recruiting
Conditions
Myotonic Dystrophy 1
Myotonic Disorders
DM1
Myotonic Dystrophy
Steinert
Dystrophy Myotonic
Steinert Disease
Myotonic Muscular Dystrophy
Myotonia
Myotonic Dystrophy Type 1 (DM1)
Interventions
Drug: AOC 1001 (del-desiran)
Drug: Placebo
Registration Number
NCT06411288
Lead Sponsor
Avidity Biosciences, Inc.
Brief Summary

A Phase 3 Randomized, Double-Blind, Placebo-Controlled, Global Study to Evaluate the Efficacy and Safety of Intravenous Delpacibart Etedesiran (abbreviated del-desiran, formerly AOC 1001) for the Treatment of Myotonic Dystrophy Type 1

Detailed Description

The study consists of a Screening Period of up to 6 weeks and 54-week Treatment Period. The anticipated duration is approximately 60 weeks.

Participants will be randomized to receive an intravenous infusion of either del-desiran or placebo at the clinical study site every 8 weeks for a total of 7 doses. The final dose will occur at Week 48, followed by a final assessment at Week 54.

After completion of Week 54 assessments, eligible participants will have the option to enroll into an open label extension (OLE) study, pending regulatory approval.

An Independent Data Monitoring Committee (IDMC) comprised of members independent and external to the Sponsor will review safety, tolerability, and efficacy (as needed) data of this study at regular intervals.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
150
Inclusion Criteria
  • Clinical and genetic diagnosis (CTG repeat ≥ 100) of DM1
  • Ability to walk independently (orthoses and ankle braces allowed) for at least 10 meters at screening

Key

Exclusion Criteria
  • Breastfeeding, pregnancy, or intent to become pregnant during the study
  • Unwilling or unable to comply with contraceptive requirements
  • Abnormal lab values, conditions or diseases that would make the participant unsuitable for the study
  • Diabetes that is not adequately controlled
  • History of decompensated heart failure within 3 months of screening. Participants with preexisting pacemaker/ICD are not excluded.
  • Body Mass Index > 35 kg/m2 at Screening
  • Recently treated with an investigational drug or biological agent
  • Treatment with anti-myotonic medication within 5 half-lives or 14 days of baseline, whichever is longer, prior to baseline.

Note: Additional protocol defined Inclusion and Exclusion criteria apply

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Del-desiranAOC 1001 (del-desiran)Del-desiran (AOC 1001) will be administered seven times
PlaceboPlaceboSaline will be administered seven times
Primary Outcome Measures
NameTimeMethod
Hand functionThrough Week 30

video Hand Opening Time (vHOT)

Secondary Outcome Measures
NameTimeMethod
Hand grip strengthThrough Week 30

by dynamometer

Myotonic Dystrophy Type 1 activity and participation scale cThrough Week 30
Quantitative Muscle Testing composite scoreThrough Week 30

by dynamometer

Trial Locations

Locations (34)

Stanford University

🇺🇸

Stanford, California, United States

University of Colorado

🇺🇸

Denver, Colorado, United States

University of Florida

🇺🇸

Gainesville, Florida, United States

University Research Center of South Florida

🇺🇸

Tampa, Florida, United States

Indiana University (IU)

🇺🇸

Indianapolis, Indiana, United States

Kansas University Medical Center

🇺🇸

Kansas City, Kansas, United States

Kennedy Krieger Institute

🇺🇸

Baltimore, Maryland, United States

Beth Israel Deaconess Medical Center

🇺🇸

Boston, Massachusetts, United States

University of Minnesota

🇺🇸

Minneapolis, Minnesota, United States

University of Rochester Medical Center

🇺🇸

Rochester, New York, United States

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Stanford University
🇺🇸Stanford, California, United States

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